Acute Necrotizing Hemorrhagic Leukoencephalitis

Scientific BACKGROUND: Acute necrotizing hemorrhagic leukoencephalitis (ANHL) is a rare, demyelinating disease of white matter, characterized with fibrinoid necrozis. Headache, fever, muscle weakness, seizure and consciousness disorders are common neurologic findings. Viral disease or infections are the most common accused etiological factors of ANHL but it is described as a fatal immune response in pathogenesis. Intracranial patology is usually asymmetric white matter lesions in periventriculler area that is not attending to grey matter. The lesions are demyelinating, fibrinoid necrosiz and edema pattern. In this report, we are discussing an ANHL case that we follow up in Ege University Medical School Hospital Neurologial Intensive Care Unit (ICU) because of its rarety, diagnostic brain biopsy material and good clinical responce to the immune supressive therapy.
CASE: A 42 old year woman presented with instability, mental diversification, speech disorder from emergency room to neurological ICU. She was taking antibiotherapy because of otitis media. During the clinical observation her neurological status progressed, she became comatose with localizing pain in her left sight on the second day in ICU. Lumbal puncture demonstrated normal opening pressure and biochemical, serological, bacteriological and cytological examinations of cerebrospinal flood (CSF) were within normal limits. Her cranial MR T1, flair, diffusion weighted images showed bilateral, diffuse hyperintense white matter lesions without contrast enhancement. Her brain biopsy demonstrated leukoencephalitis with hemorrhagic areas. The clinical and radiological regression became after using high dose steroid (1 gr/ day) therapy for ten days. During the third month of her follow-up, she had secondary generalised focal seizures which were controlled after valproic acid infusion (20 mg/kg) and levatirasetam 1000 mg/day therapy. Her last cranial MRI showed prominent regression of the lesions. She was awake, opening her eyes to verbal stimulus, had an emotional cooperation with her eyes and quadriparetic at discharge before her referral to the Rehabilitation Clinic.
CONCLUSION: It must be kept in mind that in ANHL, which is said to be extremely rare and fatal in literature, early diagnosis and high dose metil-prednisolone therapy could be life-saving.